TABLE 11-1
Types of Collagen, Their Distributions in Tissues and Properties
Type*
Composition
Distribution in Tissues
Examples of Known Disorders
I
[cd(I)]
2
a
2
«1(1)3
Bone, Tendon, Skin, Dentin,
Fascia, Arteries
Osteogensis Imperfacta (01) and Ehlers-Danlos Syndrome (EDS).
Both syndromes are clinically heterogeneous due to genetic defects
that affect the biosynthesis, assembly, postranslational modification,
secretion, fibrillogenesis, or other extracellular matrix components.
II
[O
' 1 (II)]3
Cartilage, Vitreous humor
Chondrodysplasia: Spondyloepiphysial dysplasia, a chondrogenesis
and Stickler Syndrome
III
[al(III
) ] 3
Skin, Blood vessels, Uterus
EDS Type IV: Mutation affects synthesis, structure or secretion
IV
[al(IV)]
2
a
2
(IV) other forms
are also known
Basement membrane
Alport Syndrome: characterized by nephritis and sensorineural
deafness
V
[al(V
) ] 3
[(al(V)
2
)]
2
a
2
(V)
al(V)a
2
(V)a
3
(V)
Skin, Placenta, Blood vessels,
Chorion uterus
VII
[a(VII
) ] 3
Anchoring fibrils
Epidermolysis bullosa
VIII
Not yet known
Cornea, Blood vessels,
Network-forming collagen
IX
[al(IX)a
2
(IX)a
2
(IX)
Cartilage, Fibril associated
collagen
X
[al(X
) ] 3
Cartilage
XI
a
1
(XI)a2(XI)a
1
(II)
Cartilage
XII
[al(XII
) ] 3
Soft tissues
»Collagen Types I, II, III, V, and XI are fibrillar.
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